Factors associated with health-related quality of life in children with sickle cell disease.

Abstract

Background: Sickle cell disease is an inherited haematological condition with life-threatening consequences. It can affect all aspects of the lives of children with the condition, including biopsychosocial and cognitive aspects. These children tend to have a low health-related quality of life (HRQoL).

Aim: To identify factors associated with HRQoL in Omani children with sickle cell disease.

Method: The study was a secondary analysis of data from a randomised controlled trial conducted with 72 parent-and-child dyads who were recruited from two tertiary hospitals in Oman. The aim of the original study was to examine the effects of an educational programme on the knowledge and self-efficacy of parents of children with sickle cell disease. As part of that study, parents and children completed two questionnaires on HRQoL, one generic and one specific to sickle cell disease.

Results: Parents' knowledge of sickle cell disease, parents' self-efficacy in managing their child's symptoms, parents' age, children's age and treatment with hydroxyurea were found to affect children's HRQoL.

Conclusion: Healthcare providers need to include biopsychosocial and cognitive aspects of HRQoL in their assessments of children with sickle cell disease. Programmes designed to enhance parents' and children's knowledge and self-efficacy, as well as measures designed to ensure that children receive treatment with hydroxyurea, are likely to improve the HRQoL of children with sickle cell disease.